Sickle cell anemia is a major public health problem affecting a significant portion of the African-American population. The cause of sickle cell anemia is the substitution of valine for glutamic acid in (beta)-globin. Although this has been known since 1957, it was not until 1995 that for the first time an effective therapy, hydroxyurea, for reducing painful episodes in severely affected adults with sickle cell anemia was clinically shown. Hydroxyurea belongs to a group of medicines referred to as antimetabolites. It appears to increase the flexibility of sickled cells.
Hydroxyurea has been found to decrease the incidence of sickle cell pain crisis and the acute check syndrome. These effects are at least in part related to increase hemoglobin F production by erythroid progenitors from sickle cell patients.
However, it has been observed that hydroxyurea has the tendency to suppress the bone marrow's ability to make red blood cells, white blood cells and platelets.